Treatments for IPF (Idiopathic Pulmonary Fibrosis) – 3

Long Term Oxygen Therapy (LTOT):
In 20%, IPF guidelines ‘Oxygen Therapy’, or supplementary oxygen for home use, became a strong recommendation for use in those patients with clinically significant resting hypoxemia.

Although oxygen therapy has not been shown to improve survival in IPF some data indicate an improvement in exercise capacity.

Pulmonary Rehabilitation:
Fatigue and loss of muscular mass are common and disabling problems for patients with IPF.

Pulmonary rehabilitation may alleviate the over symptoms of IPF ad improve functional status by stabilizing and reversing the extra-pulmonary features of the disease.

Palliative Care:
It focuses on reducing symptoms and improving the comfort of patients rather than treating the disease.

This may include treatment of worsening symptoms with the use of chronic Opioids for severe dyspnea and cough.
Further oxygen therapy may be useful for palliation of dyspnea in hypoxemia patients.

Palliative care also includes relief of physical and emotional suffering and psychosocial support for patients and care givers.

With disease progression patients may experience fear, anxiety and depression.
Psychological counseling should be considered.

In selected cases of particularly severe dyspnea morphine could be considered. It reduces dyspnea, anxiety and cough.

Management and follow up:
IPF is often misdiagnosed when physiological or imagine data suggest the presence of ILD leading to delay in accessing appropriate care.

After diagnosis of IPF, and appropriate treatment choice according to symptoms and stage of diseases, a close follow-up should be applied.

Due to the high variable course of disease, the higher incidence of complications like lung cancer, a routine evaluation every 3 to 6 months, including spirometry (body plethysmography) diffusion capacity testing, chest x-ray, 6MWT, assessment of dyspnea, quality of life, oxygen requirement is mandatory.

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